Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia
Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia
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DOI：
10.1046/j.1365-2141.2000.02376.x
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发表时间：
2000-11-01
影响因子：
6.5
通讯作者：
Mannucci, PM
中科院分区：
文献类型：
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作者：
Cappellini, MD;Robbiolo, L;Mannucci, PM
尽管关于这些并发症总体发生频率的流行病学数据相对较少，但中型和重型地中海贫血患者的血栓栓塞现象已有描述。为了更深入地了解地中海贫血静脉血栓栓塞的风险和机制，本研究的目的是：（i）回顾性地确定一大群中型和重型地中海贫血成人在 10 年随访期间血栓栓塞事件的患病率； (ii) 在这些患者亚组中测量凝血酶和纤维蛋白溶解酶激活的敏感标志物； (iii) 寻找可能的促凝血机制。研究发现血栓栓塞事件的发生率很高，特别是在脾切除的中间型地中海贫血患者中（29%）。这些患者的血浆凝血和纤溶激活标志物水平较高。此外，来自中间型地中海贫血脾切除患者的地中海贫血红细胞和红系前体细胞产生凝血酶的能力增强。为了评估脾切除本身对促凝血活性的作用，我们评估了因创伤而被脾切除的健康个体形成凝血酶的能力。他们产生的凝血酶量与未切除脾脏的对照组相同。总之，本研究的结果表明，脾切除的中间型地中海贫血患者存在高凝状态，并且他们的红细胞和红细胞能够在凝血酶生成中充当活化的血小板。
Thromboembolic phenomena have been described in patients with thalassaemia intermedia and major, although there are relatively few epidemiological data on the overall frequency of these complications. To obtain more insight into the risk and mechanism of venous thromboembolism in thalassaemia, the aims of this study were: (i) to establish retrospectively the prevalence of thromboembolic events in a large group of adults with thalassaemia intermedia and major during a follow up period of 10 years; (ii) to measure in subgroups of these patients sensitive markers of activation of coagulation and fibrinolysis enzymes; and (iii) to look for possible procoagulant mechanisms. A high prevalence of thromboembolic events was found, particularly in splenectomized patients with thalassaemia intermedia (29%). These patients had high plasma levels of markers of coagulation and fibrinolysis activation. Furthermore, thalassaemic red cells and erythroid precursors from splenectomized patients with thalassaemia intermedia had an enhanced capacity to generate thrombin. To evaluate the role of splenectomy per se on procoagulant activity, we evaluated the capacity to form thrombin in healthy individuals who had been splenectomized for trauma. They produced the same amount of thrombin as non-splenectomized controls. In conclusion, the results of this study show the existence of a hypercoagulable state in splenectomized patients with thalassaemia intermedia and that their red and erythroid cells are capable of acting as activated platelets in thrombin generation.